‘Nigerian Journal of Clinical Practice
March 2010 Vol. 13(1):98-100
‘Case Report
DEXTROCARDIA WITH SITUS VISCERUM INVERSUS TOTALIS IN A 65 YEAR
OLD MAN: A CASE REPORT
SA Isezuo , **SM Ma'aji, *U Isah, *A Makusidi
Departments of *Medicine and **Radiology, Usmanu Danfodiyo University Teaching Hospital, Sokoto,
Nigeria,
ABSTRACT
We report a 65 year old hypertensive and type 2 diabetic low income male observed during physical
examination to have right sided cardiac apex and heart sounds. Radiologic, electrocardiography,
‘echocardiography and computed tomogram scan studies confirmed dextrocardia with situs viscerum inversus
totalis, There was neither coexisting congenital cardiac anomaly nor disconcordant atrio-ventricular
connection. In spite of being diabetic and hypertensive, he had no evidence of ischaemic heart disease. The
blood pressure and blood glucose were adequately controlled using appropriate agents. This case, to our
knowledge, may be the documented oldest Nigerian with situs inversus dextrocardia, and shows that
individuals with this rare thoracie-abdominal organ developmental anomaly may have normal life expectancy
milar to the general population.
Key Words: Dextrocardia, situs inversus.
CASE REPORT
A65 year old low income male Nigerian presented in
the out patient medical unit of our hospital with 3
‘months history of orthopnoea, paroxysmal nocturnal
dyspnoea, dry cough, and leg and abdominal
swelling. The identified risk factors of
cardiovascular disease included type 2 diabetes
‘mellitus and systemic hypertension diagnosed 1 and
2 years earlier, respectively, and 10 pack-years of
cigarette smoking, There was no history of recurrent
upper respiratory tract infection. He is married to 2
‘wives and has 12 children. His senior sister has also
been on treatment for type 2 diabetes. Physical
examination showed evidence of biventricular
failure, He had raised blood pressure (140/100
mmHg) and features of long standing hypertension
including thickened arterial wall, locomotor
brachialis and grade II_Keith-Wagener-Baker
hypertensive retinopathy. ‘The apex beat and heart
sounds were on the right side of the chest. There was
loss of sensation to light touch involving the feet and
distal one quarter of both legs (“stocking
distribution”). The body mass index was within
‘normal range (20.0Kg/m’).
Chest radiograph (Figure 1) showed cardiomegaly
(cardiothoracic index = 58.4%) with left ventricular
preponderance and upper lobe vessel diversion. The
cardiac apex, aortic arch and gastric bubble were on
the right side of the chest. The left hemi-
diaphragmatic dome was higher than the right by
about 3.5 cm. Abdominal ultrasound showed that the
Correspondence: Prof SATsezuo
Email: simeonisezuo@yahoo.com
(Accepted 28 October 2008)
liverand the spleen were normal but located in the left
and right upper abdomen, respectively. Left sided
electrocardiogram (ECG) (Figure 2a) demonstrated
negative P wave deflection in leads I and aVL with
wave regression in the praecordial leads - mirror
image of normal. Though the P waves were inverted
in lead I and aVL of the right sided ECG, R wave
progression in the praecordial leads was normal. The
right sided ECG also show T wave inversion in leads
V,-V, Both tracings showed ‘low QRS wave
amplitude. Echocardiography done using right sided
chest echo windows confirmed the location of the
heart in the right hemithorax. Abnormal
echocardiography findings included thickened left
ventricular wall, ejection fraction of 30%, and mitral
and tricuspid ‘valve regurgitation, There were
however neither co-existing congenital cardiac/great
vessel anomaly nor abnormality of valve
morphology, myocardial wall and segmental motion.
Computed tomogram scan also confirmed the
location of the heart in the right hemithorax, and
reversal of the normal positions of the abdominal
viscera (stomach, liver, gall bladder and spleen)
(Figure 3). These thoracic and abdominal organs
were structurally normal. Apart from elevated fasting,
blood glucose (12.9 mol/L), urinalysis and blood
chemistry including electrolytes, creatinine, lipid
profiles and cardiac enzymes (Aspartate
Transaminase and Myocardial Bound Creatinine
Kinase) levels were normal.
‘A diagnosis of hypertensive diabetic heart disease
with systolic dysfunction in a patient with
dextrocardia and situs viscerum inversus totalis was
made. He was counselled and advised to cease
98cigarette smoking, The heart failure responded
satisfactorily to anti-heart failure medications
including loop diuresis, angiotensin converting
enzyme and aldosterone inhibition, and low dose
beta blockade with carvidilol. Blood sugar was
adequately controlled using metformin and
glibenclamide. Blood pressure control was achieved
without additional antihypertensive medication.
Figure l: Chest X-Ray Showing the Cardiac Apex
Pointing Towards the Right Side of the Chest,
Figure 2: (a) Left and (b) Right Sided
Electrocardiography.
Nigerian Journal of Clinial Practice March 2010, Vol.13(1)
Figure 3: Computed Tomogram Scan Showing
(a) Dextrocardia and (b) Abdominal Visceral Situs
Inversus.
a b
LEGEND
AO: Aorta, GB: Gall bladder, LA: Left Atrium
LY : Left ventricle, RA: Right atrium
RV : Right ventricle
DISCUSSION
Dextrocardia is a rare developmental anomaly in
which the primitive heart loop folds to the left in a
mirror image of a normal bulboventricular loop. This
result in the positioning of the heart in the right side of |
the thorax with the cardiac long axis directed to the
tight and inferiorly. Dextrocardia occurs in about I
pet 10, 000 in the general population '. This is lower
than an incidence rate of 1 in 12,019 pregnancies
reported in a retrospective hospital-based study
involving 85 cases of dextrocardia *. Though the
exact cause is unknown, dextrocardia has been linked
with a number’ of factors including autosomal
recessive gene with incomplete penetrance, maternal
diabetes, cocaine use, and conjoined twinning “. In
addition to being diabetic the current case report has
family history of diabetes though this does not
necessarily suggest a cause and effect relationship.
The diagnosis of dextrocardia is made usit
combination of clinical, radiological,
clectrocardiography and echocardiography findings.
Situs inversus dextrocardia may be diagnosed
incidentally during routine medical examination or
evaluation for unrelated conditions as in the current
case report.
‘There are no data on the incidence of congenital
cardiac malposition in Nigeria. Of the five cases of
dextrocardia that has been documented, to the
authors’ knowledge, in Nigeria, two had no co-
99
Situs Viscerum Inversus Isezuo et alExisting cardiac anomaly and were asymptomatic;
while one and two of the remaining three had cardiac
and noncardiac, symptomatic malformations,
respectively. Electrocardiography is an
important and interesting tool in the diagnostic
Armamentarium of dextrocardia. Reversed arm
ECG may be misdiagnosed as dextrocardia because
the QRS and P waves are negative in lead I in both
conditions (Figure 2 and 2b). However, QRS wave
progresses normally in the praccordial leads in
reversed arm ECG (Figure 2b) while it regresses in
dextrocardia (Figure 2a). Echocardiography is
required for precise analysis of cardiac anatomy as
well as detection of associated congenital cardiac or
great vessel malformation. Sonographic and
computed tomogram studies are required to confirm
the anatomical positions of abdominal visceral.
The arangements of the position of the abdominal
viscera in dextrocardia may be normal (situs solitus),
reversed (situs inversus), and indeterminate (situs
ambiguous or isomerism) in 32-35%, 35-39% and
26-28% of cases, respectively ". Dextrocardia with
‘a normal abdominal situs has a high incidence of
associated congenital cardiac anomalies includin
among others, transposition of great vessels, an
atrial and ventricular septal defects in 90.95% of
‘eases. On the other hand, dextrocardia with situs
inversus is associated with a lower, incidence of
congenital heart disease (0-10%) "*"*** Non-cardiac
malformations, on the other hand, occur in the same
Proportion in situs inyersus and ambiguous.” One
such non-cardiac malformation is the combination
of dextrocardia, abnormality of mucociliary
function and bronchiectasis (Kartagener's
). This rare triad has been reported in a
Nigerian child * and may be associated with male
infertility. About 46% and 48% of patients with
primary ciliary dyskinesia may hve situs solitus and
inversis totalis, respectively, """. The current case
report had neither pulmonary’ nor reproductive
abnormality and the observed concordant
atrioventricular connection has been reported in
74.3% of patients with situs inversus dextrocardia ™
‘Patents with tus inversus dextrocardia are ke
ie general population, susceptible to. acquire:
‘cardiac diseases, Though the curent ease report had
classical risk factors of ischemic heart disease, there
was no evidence of coronary artery lesion.
Nonetheless coronary artery disease has been
‘eported in patients with situs inversus dextrocardia
"The current case is to our knowledge the oldest
reported in Nigeria, and illustrates that patients with
situs inversus dexirocardia can have normal life
expectancy like the general population.
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